EVIDENCE FOR CARDIOEMBOLIC STROKE IN A CASE OF KEARNS-SAYRE SYNDROME

Background Cerebral infarction is a known complication in patients wit h mitochondrial encephalomyopathies (MELAS, MERRF, Kearns-Sayre syndro me), but the etiology in the different types remains uncertain. Case D escription A 33-year-old woman who had suffered from ophthalmoplegia, bilateral ptosis, ataxia, retinitis pigmentosa, and epilepsy since chi ldhood was diagnosed to have Kearns-Sayre syndrome. The diagnosis was confirmed by muscle biopsy when she was 17 years old. A pacemaker was implanted because of the occurrence of bradyarrhythmias when she was 2 4 years old. The patient was admitted to the hospital with left-sided hemiparesis of sudden onset due to right striatocapsular infarction. R esults of Doppler sonography of the carotid arteries were normal; howe ver, transesophageal echocardiography revealed a thrombus in the left atrial appendage. Conclusions Stroke in Kearns-Sayre syndrome is likel y to be due to cardiac embolism. Anticoagulant therapy should be consi dered even for mild forms of cardiomyopathies leading to left ventricu lar dysfunction.